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Taishi Yamamoto, Kisho Ohtani, Yoshihisa Tanoue, Akira Shiose, Hiroyuki Tsutsui, Primary pericardial mesothelioma presenting as constrictive pericarditis, European Heart Journal - Cardiovascular Imaging, Volume 20, Issue 5, May 2019, Page 602, https://doi.org/10.1093/ehjci/jey224
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A 53-year-old man who was previously diagnosed with small ventricular septal defect was transferred to our hospital due to exertional dyspnoea. Five months before, he had been diagnosed as having pericardial effusion of unknown aetiology. He had no history of occupational or incidental exposure to asbestos. Physical examination on admission showed distended jugular vein with positive Kussmaul’s sign and lower extremity oedema. Chest X-ray showed an enlarged cardiac silhouette with small pericardial effusion. Transthoracic (Panel A) and transoesophageal (Panel B) echocardiogram showed a nodular echogenic material encasing the heart with a few pericardial effusion. Computed tomography revealed extensive thickening of the pericardium encompassing the whole heart (Panel C). Right and left ventricle pressure recordings showed a dip and plateau pattern, suggestive of pericardial constriction. Cardiac uptake in gallium 67 scintigraphy was not observed (Panel D). A fluorine-18 fluorodeoxyglucose positron emission tomography (18F-FDG PET) demonstrated a diffuse intense 18F-FDG uptake in the pericardium with no abnormal uptake in other organs (Panel E). Hyaluronic acid was elevated to 3620 ng/mL in the pericardial fluid. Histological analysis of the pericardium obtained from surgical biopsy revealed neoplastic cell growth (Panel F) with immunostaining positive for calretinin (Panel G), WT-1, D2-40, and AE1/AE3, confirming epithelioid pericardial mesothelioma. Pericardiectomy was performed to relieve constriction (Panel H), and subsequent chemotherapy was undergone. Primary pericardial mesothelioma is an extremely rare cardiac malignancy with a poor prognosis. Elevated hyaluronic acid and diffuse intensive 18F-FDG-PET images suggested its presence in this case. Ao, Aorta; LA, left atrium; RA, right atrium.